Guillain–Barré syndrome (GBS) is defined as heterogeneous group of autoimmune polyradiculopathy, involving sensory, motor and autonomic nerves and is the most common cause of rapidly progressive flaccid paralysis 2.
Most cases preceded by upper respiratory tract infections or diarrhoea 1-3 weeks before its onset, most commonly caused by Campylobacter jejuni (25-40% seropositive) 1,3. Molecular mimicry with the bacterial agents is thought to cause the autoimmunity.
Other predisposing factors include recent surgery, lymphoma and systemic lupus erythematosus (SLE) 2.
Classical presentation of GBS includes symmetrical ascending muscle paresis or palsy, areflexia or hyporeflexia along with variable degree of sensory or autonomic involvement.
Several sub-types have been described including:
- acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
- axonal sub types:
- regional GBS syndromes:
Miller Fisher variant (MFS/ MFV)
- characterized by ataxia, areflexia without weakness and ophthalamoplegia
- anti GQ1b antibodies are present in most cases
- Miller Fisher variant (MFS/ MFV)
Guillain–Barré syndrome is diagnosed by combination of clinical presentation,CSF study and electrophysiological criteria.
CSF abnormalities are characterized by increase protein without pleocytosis, which is a non-specific finding, seen in many of the conditions which mimic GBS on imaging and clinically 1-2.
Nerve conduction abnormalities include slow or blocked nerve conduction, prolongation of distal latency and f-waves.
Chronic inflammatory demyelinating polyneuropathy (CIPD) is considered the chronic counterpart to GBS.
Radiologic studies are ordered to exclude other causes and in cases where nerve conduction studies and CSF examination are equivocal. MRI of the spine is most useful helps excluding other aetiologies such as transverse myelitis and compressive causes of polyradiculopathy.
It is essential that contrast be administered if the diagnosis is suspected as non-contrast sequences are essentially normal 2.
Typical findings in Guillain–Barré syndrome are nerve root thickening and enhancement surrounding the conus and extending along the cauda equina, resulting from break down in the blood brain barrier, which usually prevents enhancement 2.
The most common site of enhancement in Guillain–Barré syndrome is considered to be anterior nerve roots, although enhancement of the posterior nerve roots is also seen 2.
In the brain the facial nerve is the most commonly affected 1.
Treatment and prognosis
Guillain–Barré syndrome is primarily managed with IV immunoglobulins or plasmapharesis along with supportive measures, which can speed up recovery 1.
Typically improvement occurs after a number of weeks to months 1.
The differential is essentially that of nerve root / cauda equina enhancement:
- AIDS-related polyradiculopathy
- arachnoiditis from any cause (e.g. post operative, or post intrathecal injection)
- leptomeningeal carcinomatosis and lymphoma
chronic inflammatory demyelinating polyneuropathy (CIPD)
- actue presentation of CIPD can be similar to GBS
- difficult to differentiate in the first 6 weeks
- after 6-8 weeks GBS should be improving whereas CIDP will demonstrate chronic inflammation 4
- Lyme disease
- primary demyelinating disorders
- clinically isolated syndrome (CIS)
- radiologically isolated syndrome (RIS)
- multiple sclerosis (MS)
- neuromyelitis optica (NMO) (Devic disease)
- acute disseminated encephalomyelitis (ADEM) and acute haemorrhagic encephalomyelitis (AHEM)
- tumefactive demyelinating lesions
- transverse myelitis
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- Guillain-Barre Syndrome (GBS)
- primary demyelinating disorders
- 1. Fulbright RK, Erdum E, Sze G et-al. Cranial nerve enhancement in the Guillain-Barré syndrome. AJNR Am J Neuroradiol. 1995;16 (4): 923-5. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 2. Alkan O, Yildirim T, Tokmak N et-al. Spinal MRI findings of guillain-barré syndrome. J Radiol Case Rep. 2009;3 (3): 25-8. doi:10.3941/jrcr.v3i3.153 - Free text at pubmed - Pubmed citation
- 3. Hughes R, Cornblath D. Guillain-Barré syndrome The Lancet. 2005;366 (9497): . doi:10.1016/S0140-6736(05)67665-9
- 4. Li HF, Ji XJ. The Diagnostic, Prognostic, and differential value of enhanced MR imaging in Guillain-Barre syndrome. AJNR Am J Neuroradiol. 2011;32 (7): E140. doi:10.3174/ajnr.A2620 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Guillain Barre syndrome||✗|
|Guillain–Barré syndrome (GBS)||✗|
|Guillain-Barré syndrome (GBS)||✗|